J.F. Goodwin

Arrhythmia in hypertrophic cardiomyopathy. I: Influence on prognosis.

In order to examine the association between arrhythmia and subsequent prognosis, 72-hour ambulatory electrocardiographic monitoring was performed in 86 unselected patients with hypertrophic cardiomyopathy. During monitoring 23 patients experienced at least one episode of supraventricular tachycardia and 24 had ventricular tachycardia (of whom 10 had more than three episodes). The patients were then followed for a mean of 2.6 years (range one to four). Seven patients died suddenly. Of these, five had exhibited multiform and paired ventricular extrasystoles and ventricular tachycardia. These arrhythmias were significantly associated with sudden death whereas supraventricular arrhythmias were not. The patients who died suddenly were older and had experienced more symptoms than the survivors, and three had a family history of hypertrophic cardiomyopathy and sudden death. This experience provides the basis for the assessment of treatment in patients with hypertrophic cardiomyopathy and serious ventricular arrhythmia.

Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia.

The effect of amiodarone on survival was assessed in patients with hypertrophic cardiomyopathy and ventricular tachycardia in a drug trial with historical controls. During 1976 and 1977, 24 hour (seven) or 48 hour (79) electrocardiographic monitoring was performed in 86 consecutive patients; 24 had ventricular tachycardia and received conventional antiarrhythmic agents. Nineteen clinical, echocardiographic, and haemodynamic features were assessed. Seven patients died suddenly during follow up of three years; of these, five had continued to have ventricular tachycardia and two had no documented ventricular tachycardia. During 1978 and 1979, ventricular tachycardia was detected during 48 hour electrocardiographic monitoring in 21 of the next 82 consecutive patients with hypertrophic cardiomyopathy. They received amiodarone (150-400 mg/day, median 300); ventricular tachycardia was suppressed in all during repeat 48 hour electrocardiographic examination. Two patients died suddenly during a three year follow up, but neither belonged to the amiodarone treated group with ventricular tachycardia. The clinical and haemodynamic variables were similar in patients taking amiodarone and conventional agents. The fact that control of ventricular arrhythmia with amiodarone is significantly associated with improved survival suggests that amiodarone may prevent sudden death in patients with hypertrophic cardiomyopathy and ventricular tachycardia.

PROGNOSIS AND MORTALITY OF HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY

Abstract 119 patients with hypertrophic obstructive cardiomyopathy seen at the Royal Postgraduate Medical School, Hammersmith Hospital, in the years 1958-72 have been followed up for an average of 4·6 years. Familiar or suspected familial disease was present in 39 patients. Patients with angina pectoris as a sole complaint were found to have a better prognosis than those with dyspnœa at rest. The commonest complications were embolism (11 patients), atrial fibrillation (10 patients), arterial hypertension (5 patients), and infective endocarditis (3 patients). The intensity of the systolic murmur tended to decrease during the follow-up period. The commonest mode of death was sudden (19 patients). Young males with familial disease tended to be more prone to die suddenly than other groups. Beta-blockade, in small doses at least, does not protect patients from dying suddenly. The estimated mortality was 15% at five years and 35% at ten years. The development of atrial fibrillation may represent a natural progression in the course of the disease.

Arrhythmia in hypertrophic cardiomyopathy. II: Comparison of amiodarone and verapamil in treatment.

The antiarrhythmic effects of amiodarone and verapamil were compared in 19 patients with hypertrophic cardiomyopathy complicated by refractory arrhythmias. While verapamil did not reduce the incidence of arrhythmia, amiodarone abolished ventricular tachycardia in 10 of 13 patients and significantly reduced the number of ventricular extrasystoles. In addition, sinus rhythm was restored by amiodarone in three of five patients with longstanding atrial fibrillation.

Urgent aortic-valve replacement for acute aortic regurgitation due to infective endocarditis.

Abstract Between 1967 and 1970, ten patients with infective endocarditis had urgent aortic-valve replacement for severe and intractable heart-failure. Acute aortic regurgitation was the cause of this heart-failure, which is now the most common cause of death in patients with infective endocarditis. The syndrome of acute aortic regurgitation differs from chronic aortic regurgitation and may be difficult to recognise. It may be suspected when diastole is shorter than systole, giving a reversal of the usual cadence, when the cardiac output is clinically low, and when the murmurs are soft. Lack of considerable left ventricular enlargement judged by radiography and electrocardiography is an important additional feature. Premature closure of the mirral valve due to the high left ventricular diastolic pressure and short filling period of the ventricle is an invariable component and can be suspected clinically and confirmed by echocardiography. Accumulating experience indicates that aortic-valve replacement can be safely and effectively accomplished during the course of active infective endocarditis and that urgent valve replacement should be considered in patients who develop heart-failure due to valve destruction.

ENDOMYOCARDIAL FIBROSIS IN EUROPEANS RESIDENT IN TROPICAL AFRICA

Abstract A case of endomyocardial fibrosis (E.M.F.) in a European resident for 15 years in Eastern Nigeria is presented with clinical, haemodynamic, angiographic, and necropsy findings. From a review of twenty-three other cases of E.M.F. in Europeans, all resident in West Africa or the Congo, certain deductions can be made as to the aetiology of E.M.F.: it cannot be caused by a genetic characteristic nor by malnutrition, and there seems little reason to associate it with plantain eating. The distribution and pattern of the disease favour an infective agent as the cause.

Echocardiographic differentiation of hypertensive heart disease and hypertrophic cardiomyopathy.

The clinical differentiation of hypertensive heart disease from hypertrophic cardiomyopathy usually presents no problem but it is less clear whether an echocardiographic distinction can always be made and, if so, what those echocardiographic criteria of difference are. It can be inferred from recent publications that when echocardiographic criteria for hypertrophic cardiomyopathy are met in hypertensive subjects, both diagnoses may be made. This may be unjustified, and in order to clarify this problem that M-mode echocardiographic features of 37 patients with severe systemic hypertension were compared with those of 70 patients with hypertrophoic cardiomyopathy and normal blood pressure. Systolic anterior movement of the mitral valve and/or mid-systolic closure of the aortic valve were found in 82 per cent of patients with obstructive and 35 per cent of patients with non-obstructive hypertrophic cardiomyopathy. These features were not seen in patients with hypertension. The conventional echocardiographic features of left ventricular hypertrophy and function did not permit distinction between hypertensive heart disease and hypertrophic cardiomyopathy. The echocardiographic diagnosis of hypertensive heart disease from hypertrophic cardiomyopathy is, therefore, difficult unless systolic anterior movement of the mitral valve and/or mid-systolic closure of the aortic valve can be shown.

Effect of acute administration of propranolol on ventricular function in hypertrophic obstructive cardiomyopathy measured by non-invasive techniques.

Seventeen patients with hypertropic obstructive cardiomyopathy, were studied using non-invasive techniques before and after the intravenous injection of 5 mg propranolol. The folloeing were analysed: left ventricular ejection time index, derived from the carotid pulse and heart rate; the isovolumic relaxation time, derived from the apex cardiogram and phonocardiogram; the diastolic closure rate of the mirtal valve; and the left ventricular diameters, systolic and diastolic, both measured by echocardiography. Propranolol produced shortening of the isovolumic relaxation time, increase in both the diastolic closure rate of the mitral valve and left ventricular systolic and left ventricular diastolic diameters. These results show that propranolol increases the rate of filling and the volume of the left ventricle, indicating that an improvement in distensibility is produced by beta-adrenergic blockade.

Cine angiography of left ventricular aneurysms

The value of left ventricular angiography in the diagnosis of fibrous ventricular aneurysm is assessed in 11 patients. In true fibrous aneurysms, an area of immobility or paradoxical pulsation is seen to occupy at least half or more of the distal ventricular cavity. By contrast, the unaffected part of the ventricle is seen to contract satisfactorily. Although a fixed neck to the aneurysm is only occasionally noted, the differentiation between aneurysm and contracting myocardial muscle is quite obvious. The findings are compared with those seen in a further group of 11 patients with ischaemic heart disease, 4 of whom had abnormal ventricular contraction but no evidence of a true aneurysm. Abnormal contraction limited to the apex of the ventricle is seen angiographically in some patients after myocardial infarction. In this group of patients, inspection of the surface of the heart did not reveal an aneurysm. All patients with fibrous aneurysm in this series had evidence of extensive disease of the anterior descending branch of the left coronary artery.

SHOULD GOOD TEETH BE EXTRACTED TO PREVENT STREPTOCOCCUS VIRIDANS ENDOCARDITIS

Abstract Three cases of Streptococcus viridans endocarditis (S.V.E.) in edentulous patients are reported, adding to three already published. It has been suggested that to prevent subsequent attacks all teeth, good or bad, be extracted, if a patient has ever had an attack of S.V.E. This suggestion is not warranted, for edentulous patients do get S.V.E. and the extracting of teeth and the wearing of dentures both carry appreciable risks of causing infective endocarditis and create other problems for the patient.